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Lung function from infancy to school age in cystic fibrosis
1Department of Child Health, University of Leicester, Leicester Royal Infirmary.
Insights
Pulmonary function in infants with cystic fibrosis often appears normal but deteriorates by school age. Infant thoracic gas volume measurements can predict later lung function, highlighting the need to study external factors affecting cystic fibrosis lung health.
Area of Science:
- Pediatric Pulmonology
- Cystic Fibrosis Research
- Respiratory Mechanics
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
- Early detection and monitoring of pulmonary function are crucial for managing CF progression.
- Understanding the trajectory of lung mechanics from infancy to childhood is vital for timely intervention.
Purpose of the Study:
- To investigate pulmonary mechanics in infants and young children diagnosed with cystic fibrosis.
- To determine if pulmonary function tests conducted in infancy can predict lung status at school age.
- To establish a baseline for investigating external factors influencing lung function in pediatric CF.
Main Methods:
- 29 infants with cystic fibrosis underwent plethysmographic measurements of thoracic gas volume and airways resistance at 6 months.
- The same patients were reassessed at approximately 5 years 10 months of age.
- Maximum flow at functional residual capacity (infancy) was compared with maximum expiratory flow rates (childhood).
Main Results:
- Many patients exhibited normal pulmonary function measurements during infancy.
- A significant deterioration in pulmonary function was observed by school age.
- Infant thoracic gas volume was significantly correlated with school-age measurements; other parameters showed independence.
Conclusions:
- Pulmonary function in infants with cystic fibrosis may not accurately reflect later disease severity.
- Lung function declines significantly between infancy and school age in pediatric CF patients.
- The established relationships between infant and early childhood measurements provide a basis for future research on environmental influences.
Abstract:
The aim was to investigate pulmonary mechanics in patients with cystic fibrosis during infancy and again in early childhood to see whether infant tests predicted status at school age. Plethysmographic measurements of thoracic gas volume and airways resistance were made in 29 patients at 6 months and again at 5 years 10 months. Maximum flow at functional residual capacity was measured during infancy for comparison with maximum expiratory flow rates during childhood. While many patients had normal measurements during infancy, pulmonary function had deteriorated by school age. Thoracic gas volume at school age was significantly related to the values in infancy, but other measurements made during childhood were independent of infant values. The relations between measurements in infancy and early childhood described here provide a background against which the role of external factors on pulmonary function in young children with cystic fibrosis can be investigated.